Vulvar Malignancy in Neurofibromatosis Syndrome
Type 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibrom...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2013-01-01
|
| Series: | Case Reports in Obstetrics and Gynecology |
| Online Access: | http://dx.doi.org/10.1155/2013/217924 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832568095569346560 |
|---|---|
| author | Angela Musella Innocenza Palaia Lavinia Domenici Assunta Casorelli Angela Martoccia Pierluigi Benedetti Panici |
| author_facet | Angela Musella Innocenza Palaia Lavinia Domenici Assunta Casorelli Angela Martoccia Pierluigi Benedetti Panici |
| author_sort | Angela Musella |
| collection | DOAJ |
| description | Type 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibromas. Individuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma. Rarely, NF1 affects the genital tract, and isolated vulvar localization is extremely rare. Here is reported a rare case of a solitary neurosarcoma of the vulva in a 43-year-old woman affected by NF1 syndrome treated with surgical excision. The purpose of this case is to underline the possibility of association between NF1 and genital tract sarcoma and to suggest an accurate evaluation of rapid growth vulvar mass in this setting. |
| format | Article |
| id | doaj-art-a834d05b487347a1b118637eb2b03220 |
| institution | Kabale University |
| issn | 2090-6684 2090-6692 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Obstetrics and Gynecology |
| spelling | doaj-art-a834d05b487347a1b118637eb2b032202025-02-03T00:59:49ZengWileyCase Reports in Obstetrics and Gynecology2090-66842090-66922013-01-01201310.1155/2013/217924217924Vulvar Malignancy in Neurofibromatosis SyndromeAngela Musella0Innocenza Palaia1Lavinia Domenici2Assunta Casorelli3Angela Martoccia4Pierluigi Benedetti Panici5Department of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, ItalyDepartment of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, ItalyDepartment of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, ItalyDepartment of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, ItalyDepartment of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, ItalyDepartment of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, ItalyType 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibromas. Individuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma. Rarely, NF1 affects the genital tract, and isolated vulvar localization is extremely rare. Here is reported a rare case of a solitary neurosarcoma of the vulva in a 43-year-old woman affected by NF1 syndrome treated with surgical excision. The purpose of this case is to underline the possibility of association between NF1 and genital tract sarcoma and to suggest an accurate evaluation of rapid growth vulvar mass in this setting.http://dx.doi.org/10.1155/2013/217924 |
| spellingShingle | Angela Musella Innocenza Palaia Lavinia Domenici Assunta Casorelli Angela Martoccia Pierluigi Benedetti Panici Vulvar Malignancy in Neurofibromatosis Syndrome Case Reports in Obstetrics and Gynecology |
| title | Vulvar Malignancy in Neurofibromatosis Syndrome |
| title_full | Vulvar Malignancy in Neurofibromatosis Syndrome |
| title_fullStr | Vulvar Malignancy in Neurofibromatosis Syndrome |
| title_full_unstemmed | Vulvar Malignancy in Neurofibromatosis Syndrome |
| title_short | Vulvar Malignancy in Neurofibromatosis Syndrome |
| title_sort | vulvar malignancy in neurofibromatosis syndrome |
| url | http://dx.doi.org/10.1155/2013/217924 |
| work_keys_str_mv | AT angelamusella vulvarmalignancyinneurofibromatosissyndrome AT innocenzapalaia vulvarmalignancyinneurofibromatosissyndrome AT laviniadomenici vulvarmalignancyinneurofibromatosissyndrome AT assuntacasorelli vulvarmalignancyinneurofibromatosissyndrome AT angelamartoccia vulvarmalignancyinneurofibromatosissyndrome AT pierluigibenedettipanici vulvarmalignancyinneurofibromatosissyndrome |